title: Revised International Prognostic Scoring System (IPSS) predicts survival and leukemic evolution of myelodysplastic syndromes significantly better than IPSS and WHO Prognostic Scoring System: validation by the Gruppo Romano Mielodisplasie Italian Regional Database.
abstract: OBJECTIVE
  The definition of disease-specific prognostic scores plays a fundamental role in the treatment decision-making process in myelodysplastic syndrome (MDS), a group of myeloid disorders characterized by a heterogeneous clinical behavior.
  
  
  METHODS
  We applied the recently published Revised International Prognostic Scoring System (IPSS-R) to 380 patients with MDS, registered in an Italian regional database, recruiting patients from the city of Rome (Gruppo Romano Mielodisplasie). Patients were selected based on the availability of IPSS-R prognostic factors, including complete peripheral-blood and bone marrow counts, informative cytogenetics, and follow-up data.
  
  
  RESULTS
  We validated the IPSS-R score as a significant predictor of overall survival (OS) and leukemia-free survival (LFS) in MDS (P < .001 for both). When comparing the prognostic value of the International Prognostic Scoring System (IPSS), WHO Prognostic Scoring System (WPSS), and IPSS-R, using the Cox regression model and the likelihood ratio test, a significantly higher predictive power for LFS and OS became evident for the IPSS-R, compared with the IPSS and WPSS (P < .001 for both). The multivariate analysis, including IPSS, WPSS, age, lactate dehydrogenase, ferritin concentration, Eastern Cooperative Oncology Group performance status, transfusion dependency, and type of therapy, confirmed the significant prognostic value of IPSS-R subgroups for LFS and OS. Treatment with lenalidomide and erythropoiesis-stimulating agents was shown to be an independent predictor of survival in the multivariate analysis.
  
  
  CONCLUSIONS
  Our data confirm that the IPSS-R is an excellent prognostic tool in MDS in the era of disease-modifying treatments. The early recognition of patients at high risk of progression to aggressive disease may optimize treatment timing in MDS.
authors.affiliation: Department of Hematology, Universita' Cattolica S. Cuore, Rome, Italy. mtvoso@rm.unicatt.it
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Voso
authors.forename: Susanna
authors.initials: S
authors.lastname: Fenu
authors.forename: Roberto
authors.initials: R
authors.lastname: Latagliata
authors.forename: Francesco
authors.initials: F
authors.lastname: Buccisano
authors.forename: Alfonso
authors.initials: A
authors.lastname: Piciocchi
authors.forename: Maria Antonietta
authors.initials: MA
authors.lastname: Aloe-Spiriti
authors.forename: Massimo
authors.initials: M
authors.lastname: Breccia
authors.forename: Marianna
authors.initials: M
authors.lastname: Criscuolo
authors.forename: Alessandro
authors.initials: A
authors.lastname: Andriani
authors.forename: Stefano
authors.initials: S
authors.lastname: Mancini
authors.forename: Pasquale
authors.initials: P
authors.lastname: Niscola
authors.forename: Virginia
authors.initials: V
authors.lastname: Naso
authors.forename: Carolina
authors.initials: C
authors.lastname: Nobile
authors.forename: Anna Lina
authors.initials: AL
authors.lastname: Piccioni
authors.forename: Mariella
authors.initials: M
authors.lastname: D'Andrea
authors.forename: Ada
authors.initials: A
authors.lastname: D'Addosio
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Leone
authors.forename: Adriano
authors.initials: A
authors.lastname: Venditti
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D018450:Disease Progression; D005260:Female; D006801:Humans; D007391:International Cooperation; D008297:Male; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000175:diagnosis*; D011379:Prognosis; D012107:Research Design; D012189:Retrospective Studies; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome; D014944:World Health Organization; D055815:Young Adult